We report the 16th case of mu-chain disease. Unlike most previously reported cases, free mu-chains, but not Bence Jones protein, were present in the urine. The usefulness of immunofixation as a means of detecting free heavy chains in the serum is demonstrated.
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Iran J Allergy Asthma Immunol
December 2021
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran AND Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden.
Although the majority of monogenic defects underlying primary immunodeficiency are microlesions, large lesions like large deletions are rare and constitute less than 10% of these patients. The immunoglobulin heavy chain (IGH) locus is one of the common regions for such genetic alterations. This study describes a rare case of autosomal recessive agammaglobulinemia with a homozygous large deletion in chromosome 14q32.
View Article and Find Full Text PDFBackground: We encountered a rare case of Waldenstrom macroglobulinemia with temporary appearance of 7S IgM half molecule and with monoclonal proteins binding to agarose gel.
Methods: The patient's serum and urine were analyzed using sodium dodecyl sulfate-polyacrylamide gel electrophoresis and immunoblotting. The N-terminal amino acid sequences of the IgM with abnormal mass (68 kDa) were determined and compared with those of known immunoglobulin.
Cell Mol Immunol
January 2014
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Immunoglobulin (Ig), a characteristic marker of B cells, has been reported to be expressed in epithelial cells, with a suggested role in their growth and survival. We have previously reported that IgG heavy chain is expressed in acute myeloid leukemia (AML), but not in the monocytes or neutrophils from patients with non-hematopoietic neoplasms or healthy controls. In the present study, we assessed IgM heavy chain expression and repertoire in human myeloid cells.
View Article and Find Full Text PDFXi Bao Yu Fen Zi Mian Yi Xue Za Zhi
November 2006
Department of Laboratory Medicine, Chengdu Medical College, Chengdu 610083, China.
Aim: To evaluate the effect of FcgammaRIIB(1) (CD32) representative self-inhibitory adjustive mechanism of B cells on pathogenesis of systemic lupus erythematosus (SLE) by observing the expression characteristic and functional state of molecules on the surface of B cells from SLE patients.
Methods: The peripheral blood mononuclear cells (PBMC) were prepared by density gradient centrifugation, and the B cells were isolated from PBMC by magnetic activated cell sorting (MACS). The fluxes of intracytoplasmic calcium ([Ca(2+)](i)) of B cells activated by different activators were measured by fluorescence spectrophotometric method.
Rinsho Byori
September 2003
Department of Clinical Laboratory, Kobe City General Hospital, Kobe 650-0046.
We report a case of mu-heavy chain disease. A 56-year-old woman presented with anemia and hemorrhagic diathesis. The serum of the patient was found to have free mu-heavy chain.
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